Fowler's Syndrome

Introduction

Welcome to the Department of Uro-neurology information
page on Fowler’s Syndrome. This website was setup in response to
requests for information to be made available to patients with this
condition.

There is continuous research going on into Fowler’s
syndrome but apart from academic medical journals and some urological
centres there is no easy way to communicate what is known about this
condition to patients.

This website was setup by two research registrars at the
National Hospital of Neurology and Neurosurgery Drs. Sam Datta and
Rajesh Kavia, and put together by Sidhartha Datta. The aim of this
website is to provide information about Fowler’s Syndrome and how to
cope with the diagnosis.

Professor Clare J Fowler, MB, BS MSc FRCP is a Professor
of Uro-neurology at the National Hospital of Neurology &
Neurosurgery and first described the syndrome in 1985, she has
continued to work on patients’ problems ever since.

She has unique first hand experience in the diagnosis and
treatment of this challenging condition, but acknowledges that there is
still a lot to understand.

What is Fowler’s Syndrome?

First described in 1985, it is a cause of urinary
retention (inability to pass water normally) in young women. Urinary
retention in young women is not common but can be quite debilitating.
The abnormality lies in the urethral sphincter (the muscle that keeps
you continent). The problem is caused by the sphincter’s failure to
relax to allow urine to be passed normally. There is no neurological
disorder associated with the condition. Up to half the women have
associated polycystic ovaries.

What sort of symptoms do patients present with?

The typical woman who is seen with the condition is in her
20-30s and may infrequently pass urine with an intermittent stream. The
normal sensation of urinary urgency expected with a full bladder are
not present but as the bladder reaches capacity there may be pain and
discomfort, and she finds that she is not able to pass urine. This can
happen spontaneously or following an operative procedure
(gynaecological, urological or even ENT) or following childbirth.

Classically, the woman presents to the hospital as they
have been unable to pass urine for many hours and a catheter (tube that
drains the bladder) is inserted, and usually over a litre is drained
with consequent relief of the pain.

If the retention occurs after an operation in hospital,
urinary retention may occur during the night after the operation when
the patient develops pain over their bladder.

Initial hospital management is carried out by the urology
team at the local hospital but if the symptoms do not resolve, the
patient maybe referred on.

There is a spectrum in the severity of the condition, with
some patients being able to pass urine with difficulty but leaving
significant amounts, and some not being able to pass any (complete
retention).

Many women who are not in complete retention, may present
to they doctors complaining of recurrent cystitis (bladder infections)
or even kidney infections.

What causes these symptoms?

Most of symptoms of Fowler’s Syndrome are caused by inability to empty the urine that is stored in the bladder.

Some women may experience back pain, suprapubic pain (pain
over the bladder) or dysuria (discomfort/burning whilst passing urine)
due the urinary infections.

The cause and process which gives rise to Fowler’s Syndrome is not known and is still under research.

How do you diagnose the condition?

The key diagnostic test for the condition is a Sphincter
Electromyogram (EMG). However, this is somewhat uncomfortable since a
needle must be used to record from the sphincter and needs specialist
expertise and equipment. Other tests that may be carried out which
indicate the diagnosis is likely include flow rate, residual volume,
urethral pressure profile and ultrasound sphincter volume. These tests
depend on whether you pass urine.

What are the tests? What happens? What do they tell us?

Flow Rate

If you can pass urine, you sit normally as you would at
home but on a special toilet that measures the speed of your stream and
how long it takes you to pass urine. The computer measures the rate of
flow of urine and draws a graph. We can use this to see if your stream
is interrupted and how bad it is. The test is easy to perform and is
totally non-invasive.

Residual Volume

This test, carried out using an ultrasound scanner, gives
useful information on how well the bladder is emptying. This scan
measures the amount of urine left in your bladder after you have been
to the toilet. Normally, the bladder should be completely empty after
passing urine.

Urodynamics

This test, sometimes also called “cystometry” is more
useful if you cannot pass urine and involves placing two small
catheters (tubes), one in the bladder and one in the rectum (back
passage). The bladder is slowly filled with saline (salt water) and is
monitored for any irregular spasms. Once, the bladder is full, you are
asked to pass urine with the catheters in. This gives information on
what pressure the bladder muscle generates for a particular urine flow
rate.

This test takes 30-40 minutes and may cause a little
discomfort on insertion of the catheters. However once the catheters
are inserted, it is fairly painless.

Urethral Pressure Profile

Whilst you lie on your back, a catheter is inserted into
the urethra (similar to that used in the cystometry) and saline is
infused slowly through the catheter. The catheter is then withdrawn and
re-inserted (six times) into the bladder whilst the pressure of the
urethral sphincter is measured. This test gives information on how much
pressure is generated by the sphincter, and thus how overactive the
muscle is.

Ultrasound Sphincter Volume Measurement

The volume of the urethral sphincter (the muscle that
keeps one continent in the normal state) is measure using ultrasound. A
small probe is placed in the vagina, and the sphincter is identified.
Measurements are then made and the volume calculated. It can be a
little uncomfortable on insertion of the probe, but once the sphincter
is found, most patients do not find it too bothersome. An overactive
sphincter may enlarge due to continuous ‘muscle activity’.

Sphincter Electromyogram (EMG)

This is the gold standard test for Fowler’s syndrome, and
is done to confirm our other findings. Professor Fowler usually
performs this test within the department.

With the patient lying on their back, local anaesthetic is
injected into the sphincter region. A small needle is then used to take
recordings from the sphincter. The area from which the needle takes the
recording is very small (1mm³). (It is quite a complex and tricky test
sometimes).

Characteristic waveforms and sounds can be identified
using this technique. The abnormality in Fowler’s syndrome is a complex
repetitive discharge and decelerating bursts, but to the non
specialist, it is the sound of ‘helicopters’ and ‘whales’.

What happens to me once the diagnosis is made?

Fowler’s syndrome is a condition which is slowly being
understood. There is no absolute cure for the condition yet. The aim of
treatment is to try and ensure bladder emptying.

Bladder function may spontaneously recover in some
patients, especially in the group in whom the problems started after
childbirth.

In patients with little recovery, it can be a lifelong condition which can cause significant impact to quality of life.

At the National Hospital, we have a specialist team of
doctors, nurses and continence advisors to help manage your condition
long term. There are various treatments that are used to regain control
and overcome the symptoms.

Are there any treatments?

Currently the treatments for Fowler’s syndrome are being
researched and developed. Depending on the severity of the condition,
there are various but limited options.

Often patients have a poor urine stream but can still void
almost normally. In these patients, we monitor their residual volume.
If they are low, no intervention is necessary.

Some patients have a large residual volume which gives
rise to urinary infections and a large bladder. These patients are
helped by regular clean intermittent catherisation (putting a sterile
catheter into the bladder at regular intervals to empty the bladder).

The most severe patients, those in complete retention may
be candidates for sacral nerve stimulation, which is the only treatment
shown to restore voiding. However this requires major efforts by the
patient, is expensive, often troubled by operative difficulties and
cannot be regarded as a "good fix".

What is intermittent self catheterisation?

This is where you as the patient will insert a catheter
(tube) into your bladder to empty it. This is done at regular intervals
to ensure there is not a stagnant volume of urine, which can give rise
to infections. Our continence advisors can teach this technique, and
suggest ways to make it as easy as possible. They also can help arrange
to put you in contact with suppliers of the most suitable catheters for
your convenience.

The procedure is not too uncomfortable, however many women
do complain that although it is easy to insert the catheter, removal is
painful and that the catheter gets ‘stuck’ when attempting to withdraw
it.

What is Sacral Nerve Stimulation?

Sacral nerve stimulation (SNS) is a process whereby small
electrical pulses stimulate nerves in the lower back (just above the
tail bone [sacrum]). The nerves stimulated are those involved in
control of bladder sensation. How the stimulation works at restoring
voiding is still under research.

If you are deemed eligible for SNS, then the first stage
is to test stimulation, peripheral nerve evaluation (PNE). A temporary
lead is inserted into the 3rd sacral foramen (naturally occurring hole
in the lower spine) using an external stimulator (looks similar to
pager) and the response is assessed for 3 to 7 days. You will need to
keep a diary to record your symptoms. In approximately two thirds of
the women there will be a restoration of voiding.

If bladder function is improved then the patient will be
placed on the waiting list for a staged SNS implant. The implant is the
InterStim Therapy® produced by Medtronic® (more information about the
InterStim implant is available at www.medtronic.com).

The staged operation involves the placement of a permanent
electrode (see right), either under local or general anaesthetic. This
is connected to an external stimulator source and the response assessed
for 4 weeks. If the lead placement results in good voiding with minimal
side effects then the stimulator is internalised. It is thought that
this second longer period of test stimulation with the permanent lead
should reduce the future complications and improve success rates. The
stimulator is usually placed in the buttock or abdomen.

Why may I not be considered for SNS?

If you live a great distance form the National Hospital or
you find the journey to the hospital difficult then we may not consider
you for the treatment, as on average you would be required to be seen
at the hospital four times a year.

You may not be considered for SNS if the test stimulation is unsuccessful.

What options have I got if SNS is not possible and I cannot self catheterise?

If you are unable to catheterise and SNS is not an option,
then a long term catheter may be required. This may be a permanent tube
that drains your bladder either via your urethra or via a suprapubic
catheter (a tube placed below you belly button, into your bladder). For
more information see attached article.

Very occasionally, if you are in complete retention you
may be offered more radical procedures, such as removal of the bladder
(these may sound attractive but are not short of complications or
suitable for all).

This technique has showed some good results with many not
needing to catheterise. However it is not always easy to get a good
result and some patients have found over time that they have run into
problems with batteries needing replacement, reduced effect and needing
to catheterise or pain down the leg.